CJD Update - French Woman
May Have vCJD In 1971

From Patricia Doyle, PhD
Hello, Jeff - If they can prove there was a vCJD case in 1971 they may have to rethink origin of BSE.
Patricia Doyle
CJD (NEW VAR.), UPDATE 2005 (04)
A ProMED-mail post
ProMED-mail is a program of the
International Society for Infectious Diseases
[The UK Department of Health web-site has been revised and the monthly new variant Creutzfeldt-Jakob disease statistics are now appended to the table "Creutzfeldt-Jakob disease in the UK by Calendar Year (since 1990)" which can be accessed at
The definition of the designations deaths, definite cases, probable vCJD cases, and the case definitions can be found by accessing the Department of Health web-site or by reference to a previous ProMED-mail post in this thread (for example, CJD (new var.) - UK: update Mar 2002 20020305.3693).
The incidence of variant Creutzfeldt-Jakob disease, abbreviated CJD (new var.) or vCJD in ProMED-mail, in the UK appears to have plateaued, or perhaps to be in decline. Therefore, since many of the reports appearing in the update are only peripherally related to the situation in the UK, the opportunity is being taken to drop the designation UK from the title of this thread. - Mod.CP]
In this update:
[1] UK DH variant CJD monthly statistics - as of Mon 1 Apr 2005
[2] Susceptibility of primates to BSE
[3[ & [4] French case of variant CJD in 1971?
[5] NIH brain collection
Date: Mon 4 Apr 2005
From: ProMED-mail <
Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease
Statistics, Press release no. 2005/0161, Mon 4 Apr 2005 [edited]
Monthly Creutzfeldt Jakob Disease Statistics - As of 1 April 2005
The Department of Health is today issuing the latest information about the numbers of known cases of Creutzfeldt Jakob disease. This includes cases of variant Creutzfeldt Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD] - the form of the disease thought to be linked to BSE. The position is as follows:
Definite and probable CJD cases in the UK
Summary of vCJD Cases - Deaths
Deaths from definite vCJD (confirmed): 107
Deaths from probable vCJD (without neuropathological confirmation): 42
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 149
Summary of vCJD Cases - Alive
Number of probable vCJD cases still alive: 6
Number of definite or probable vCJD (dead and alive): 155
(The next table will be published on Monday 2 May 2005)
[Since the previous monthly statistics were released on Mon 4 Mar 2005, the number of deaths from definite vCJD has increased by one, but the total number of deaths from definite or probable vCJD remains 149. The number of probable vCJD cases still alive has increased from 5 to 6. Therefore the overall total number of definite or probable vCJD cases (dead and alive) has increased from 154 to 155.
As of 1 Apr 2005, so far in the UK for the year 2005 there have 25 referrals of suspected CJD; and there have been 9 deaths from sporadic CJC, one each from GSS (Gerstmann-Straussler-Scheinker syndrome) and vCJD, and none from familial CJD or iatrogenic CJD. - Mod.CP]
Date: Mon 14 Mar 2005
From: ProMED-mail <
Source: Lancet online, 27 Jan 2005; 365: 781-83, 2005 [edited]
Susceptibility of Primates to BSE
[A paper entitled "Risk of oral infection with bovine spongiform encephalopathy agent in primates," by Corinne Ida Lasmezas and 12 others,appeared in Lancet at the end of January 2005. The abstract is reproduced below. ProMED-mail regrets the delay in posting this information submitted by a correspondent- Mod.CP]
Abstract: "The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE) -- which can lead to variant Creutzfeldt-Jakob disease (vCJD) -- is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave 2 macaques a 5-g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man."
[Reassuring or not? - Mod.CP]
Date: Thu 24 Mar 2005
From: ProMED-mail <
Source: The Washington Times, United Press International, Thu 24 Mar 2005
French Woman May Have Had Variant Creutzfeldt-Jakob Disease in 1971
The brain of a French woman who died in 1971 shows evidence consistent with human mad cow disease, United Press International (UPI) has learned, a finding that if confirmed would indicate the deadly disease began infecting people more than 20 years earlier than previously thought.
A former National Institutes of Health scientist said he tested the woman's brain in 2000 and it showed a pattern that looked like variant Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD] -- a fatal, brain-wasting illness humans can contract from eating beef products infected with the pathogen that causes mad cow disease, also known as bovine spongiform encephalopathy or BSE.
vCJD was unheard of in 1971. The 1st recognized case was detected in the United Kingdom in 1995, so if the French woman did indeed suffer from vCJD, the case would shift the origins of the disease back more than 2 decades and possibly to a different country. The woman's brain is held at the National Institutes of Health in Bethesda, Maryland.
"Variant CJD could've been around for donkey's years, who knows?" said Bruce Johnson, a former researcher at the NIH's Laboratory for Central Nervous System Studies (CNSS), who examined the woman's brain. The CNSS lab received brains from CJD patients from all over the world and has samples dating back to 1963. The woman's identity could not be revealed for confidentiality reasons, but it is known she was French and approximately 40 to 50 years old when she died in 1971, Johnson said.
Johnson told UPI he tested the woman's brain using a technique called Western blotting, which can detect prions -- the infectious agents thought to play a role in causing vCJD and similar diseases. At the time of her death, the woman was thought to be suffering from sporadic CJD, a condition with no known cause that appears to arise spontaneously. However, Johnson said, the prions he detected looked different from those associated with [sporadic] CJD and instead were consistent with the prion strain associated with vCJD. The pattern on the test "was more like BSE than CJD," Johnson said, noting he never saw a pattern like that in the hundreds of other brains from CJD patients he had tested.
A sample of the woman's brain had been injected into a chimpanzee sometime around 1977, and when Johnson examined the chimpanzee's brain, it, too, showed a pattern consistent with vCJD -- not sporadic CJD. "So she may have been an early case of BSE in France before it ever got to England," he said.
Johnson said he never published his finding because he wanted to confirm it, but he never had an opportunity to do so before he retired in 2003. The CNSS lab was officially closed in April 2004. He said he hopes to conduct further examinations of the woman's brain when he starts a new position with the Food and Drug Administration. "If we've still got her brain, we can look and see if it's BSE," he said. One possible way is to inject some of the woman's brain into mice.
BSE first showed up in humans in the United Kingdom beginning around 1995. In all, 154 [now 155] people in that country have been infected with the human equivalent of mad cow disease. France runs a distant second in vCJD cases with nine. A recent report published in the journal "Veterinary Research" estimated that from 1980 to 2000 more than 300 000 cattle were infected with BSE in France, yet went undetected. Stephen Dealler, a microbiologist at Lancaster Royal infirmary, recently proposed a hypothesis that some of the people who developed vCJD in the United Kingdom may have been exposed to BSE in baby food beginning as early as 1970.
Johnson subscribes to the hypothesis put out by his NIH colleague Joe Gibbs, who thought it was possible that all mammals, including cows, spontaneously develop a mad cow-like disease at the rate of one per million. If that is true, Johnson said, the French woman may have developed her condition from being unfortunate enough to have eaten infected meat from that one in a million animal.
Dr. Paul Brown, former medical director of the CNSS lab and an expert on CJD and BSE, worked with Johnson. He told UPI he remembered Johnson mentioning the French woman's brain, but the information did not sound conclusive. He said more research would need to be done to determine if the woman's disease was vCJD, including injecting it into laboratory animals and having CJD experts examine the brain tissue.
Patient advocacy representatives had mixed reactions. "I would be looking to get the opinion of more than one CJD neurologist before making any further comment," Graham Steel, vice-chair of the patient advocacy group Human BSE Foundation in the United Kingdom, told UPI. "It doesn't surprise me at all that you can find a vCJD case in the NIH's brain collection," said Terry Singeltary, who is associated with several CJD patient groups and closely monitors developments about these diseases. "It wouldn't surprise me for it to go back that far," Singeltary, told UPI. "A lot of scientists believe this BSE epidemic started way before 1984."
Johnson said it was possible there could be other vCJD cases in the NIH's collection, which consists of brain samples from hundreds of patients thought to have CJD. That may never be known, however. The brains have never been screened for vCJD and the NIH may destroy part or all of the collection.
[Byline: Steve Mitchell]
Date: Thu 31 Mar 2005
From: Terry S. Singeltary Sr. <
Source: The Washington Times, United Press International, Thu 31 Mar 2005
French re-testing 1971 case for vCJD
French researchers are re-analyzing the brain of a woman who died in 1971 for possible variant Creutzfeldt Jakob disease (vCJD), the human version of mad cow, United Press International (UPI) has learned. If the woman did have vCJD it would suggest the deadly brain-wasting illness began infecting people more than 20 years earlier than previously thought. The 1st recognized case of vCJD, which humans can contract from eating beef products contaminated with the mad-cow pathogen, was seen in 1995 in the United Kingdom. As first reported by UPI last week, a former scientist for the U.S. National Institutes of Health in Bethesda, Maryland, said he conducted a test on the woman's brain in 2000 and saw patterns that indicated possible vCJD, rather than sporadic CJD -- a similar condition that has no known cause.
"We have been very recently informed of the statement of this scientist and we have been able to identify the case," Dr. Annick Alperovitch, leader of the French participation in The European and Allied Countries Collaborative Study Group of CJD, wrote in an e-mail to UPI. "Available data and material will be carefully re-analyzed, but this will take quite (some) time," added Alperovitch, who also holds a position with Institut National de la Sante et de la Recherche Medicale (INSERM) near Paris. She offered no further details.
"This is potentially earth-shattering,"Neil Cashman, a vCJD expert at the University of Toronto, told UPI. "It's like finding a case of autopsied AIDS back in the 1700s." More than 160 cases of vCJD have occurred worldwide, with nearly all appearing in the United Kingdom where there was a massive outbreak of mad cow disease starting in the 1980s. 9 vCJD cases have been detected in France, which also experienced an outbreak of mad cow.
Bruce Johnson, the former NIH scientist, said he used a technique called western blotting to analyze the woman's brain for prions -- abnormal proteins thought to play a role in causing CJD and vCJD. A western blot test revealed a prion strain that looked more like it belonged to vCJD than CJD, he said, and added he had obtained a similar pattern from a chimpanzee that had been inoculated with the woman's brain. Further tests that could be conducted on the French woman's brain to confirm which type of disease she had contracted include inoculating a sample of the brain into mice or non-human primates, Cashman said. The mice tests can take about one year, however.
Patient-advocacy groups applauded the decision of French researchers to reopen the case. "I'm glad they're going to look at it," said Terry Singeltary, who lives in Bacliff, Texas, and is involved with several groups composed of families of patients who developed CJD or vCJD. Singeltary wanted more than one lab to examine the specimens to provide confirmation and ensure accuracy of the testing. "I surely would like someone else to look at the brain besides the French government," he told UPI. Singeltary said he would like to see a re-examination of certain U.S. patients who were diagnosed with CJD.
[Byline: Steve Mitchell]
Date: Fri 1 Apr 2005
From: Terry S. Singeltary Sr. <
Source: The Washington Times, United Press International, Fri 1 apr 2005
Groups Seek to Save NIH Brain Collection
Scientists, consumer groups, and patient-advocates have embarked upon efforts -- including petitioning members of Congress and seeking storage space at a Canadian university -- to prevent the National Institutes of Health (NIH) from destroying an irreplaceable collection of human brains from patients afflicted with a condition similar to mad cow disease.
As United Press International (UPI) reported last week, the NIH has begun shopping for a new home for its collection of brains, spinal fluid and other tissues from hundreds of patients around the world who died from Creutzfeldt Jakob disease (CJD) -- an incurable, fatal, brain-wasting illness. The collection dates back to 1963 and the consensus among scientists in this field is it is invaluable for research and could provide insights that might aid in developing diagnostic tests, treatments or cures for CJD.
NIH officials, however, maintain the remaining samples in the collection -- stored in some 30 freezers by the National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- are of little value and may be disposed of if researchers or institutions do not come forward to claim them. Families of patients who died of CJD have reacted with outrage, concerned that the effort mounted to collect the brains in the 1st place has been all for naught. Several have contacted their respective members of Congress and urged them to step in.
"The brains and brain tissue were sent to NIH in good faith for future research and destroying them is an outrage," Terry Singeltary, a patient advocate in Bacliff, Texas, wrote in a letter to Sen. Kay Bailey Hutchinson, R-Texas, and several other members of the state's congressional delegation.
CJD belongs to a group of diseases -- called transmissible spongiform encephalopathies or TSEs -- that includes mad cow disease, chronic wasting disease in deer and elk, scrapie in sheep and several types of CJD in humans. There is no cure for CJD and it typically results in death within a year after the onset of symptoms. Consumer groups also are concerned and are considering taking steps to ensure the brain collection will be preserved. "This is outrageous," Michael Hansen, a biologist and senior research associate with Consumers Union in Yonkers, N.Y., told UPI. "Those brains are a critical resource for CJD science and they must be at a research facility.
How can we claim to be a scientific country if we're going to be throwing away an irreplaceable repository of the 1st evidence of these diseases?" asked Felicia Nestor, who serves as a consultant to Public Citizen.
There may be hope yet for the collection, however. Neil Cashman, an expert on TSEs at the University of Toronto's Center for Research in Neurodegenerative Diseases, told UPI he has been attempting to drum up support for acquiring the collection with his colleagues at the University of British Columbia in Vancouver -- where he plans to move this summer. "The goal would be to make it a resource for the world and make the tissues available to scientists who had a reasonable request," he added.
One brain in the collection, that of a French woman who died in 1971, may help provide clues about the origins of variant CJD -- a condition similar to CJD that humans can contract from eating beef products contaminated with the mad-cow pathogen. The 1st recognized case of vCJD occurred in 1995 in the United Kingdom, but an NIH scientist said he tested the French woman's brain in 2000 and found signs consistent with vCJD -- not CJD. French researchers currently are re-examining specimens from the case to determine if the woman was indeed infected with vCJD. If she was, it would suggest the disease began infecting people more than 20 years earlier than previously thought. Cashman said the case underscores the value of the NIH brain collection. "There is information locked up in these freezers that will be lost forever if this collection is destroyed," he said.
Byline: Steve Mitchell
Patricia A. Doyle, PhD
Please visit my "Emerging Diseases" message board.
Zhan le Devlesa tai sastimasa
Go with God and in Good Health



This Site Served by TheHostPros